Therapy-related myeloid leukemia following Pleuropulmonary blastoma: A case report

The development of secondary malignancy (SM) is the most worrisome long-term complication of childhood cancer. Acute myeloid leukemia is the most prevalent neoplasm that occurs after treatment with alkylating agents and topoisomerase II inhibitors. Pleuropulmonary blastoma (PPB) is a rare lung neoplasm in children. Type II and type III of this cancer are markedly aggressive and have a recurrent nature. Chemotherapy, radiation therapy, and hematopoietic stem cell transplant (HSCT) are treatment modalities that make these patients prone to secondary malignancy. Here was presented and discussed a case of myeloid leukemia 3.5 years after treatment of Pleuropulmonary blastoma in a 5.5-year-old boy who was a candidate for high dose chemotherapy and autologous stem cells transplant (auto-SCT) because of frequent recurrence and lack of response to chemotherapy and radiation therapy. It seems this is the first reported case of therapy-related myeloid leukemia (t-AML) after PPB in children. Awareness of the creation of this complication following administration of cytotoxic therapies in the treatment of solid tumors will increase physician attention in the selection of treatment modality as well as the counseling of patients at the time of diagnosis.

Lung Ultrasound for Imaging of B-Lines in Dogs and Cats—A Prospective Study Investigating Agreement between Three Types of Transducers and the Accuracy in Diagnosing Cardiogenic Pulmonary Edema, Pneumonia and Lung Neoplasia

Transthoracic heart and lung ultrasound (LUS) was performed in 200 dogs and cats with dyspnea to evaluate the agreement between the results obtained using three types of transducers (microconvex, linear, and phased array) and to determine the accuracy of LUS in discriminating between three conditions commonly causing dyspnea in companion animals: cardiogenic pulmonary edema (CPE), pneumonia, and lung neoplasm. The agreement beyond chance was assessed using the weighted Cohen’s kappa coefficient (κw). The highest values of κw (>0.9) were observed for the pair of microconvex and linear transducers. To quantify B-lines the lung ultrasound score (LUSscore) was developed as a sum of points describing the occurrence of B-lines for each of 8 standardized thoracic locations. The accuracy of LUSscore was determined using the area under ROC curve (AUROC). In dogs AUROC of LUSscore was 75.9% (CI 95%: 65.0% to 86.8%) for distinguishing between lung neoplasms and the two other causes of dyspnea. In cats AUROC of LUSscore was 83.6% (CI 95%: 75.2% to 92.0%) for distinguishing between CPE and the two other causes of dyspnea. The study shows that results obtained with microconvex and linear transducers are highly consistent and these two transducers can be used interchangeably. Moreover, the LUSscore may help identify dogs with lung neoplasms and cats with CPE, however its diagnostic accuracy is only fair to moderate.

DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma

DICER1 syndrome (OMIM 606241, 601200) is a rare autosomal dominant familial tumor predisposition disorder with a heterozygous DICER1 germline mutation. The most common tumor seen clinically is the pleuropulmonary blastoma (PPB), a lung neoplasm of early childhood which is classified on its morphologic features into four types (IR, I, II and III) with tumor progression over time within the first 4–5 years of life from the prognostically favorable cystic type I to the unfavorable solid type III. Following the initial report of PPB, its association with other cystic neoplasms was demonstrated in family studies. The detection of the germline mutation in DICER1 provided the opportunity to identify and continue to recognize a number seemingly unrelated extrapulmonary neoplasms: Sertoli-Leydig cell tumor, gynandroblastoma, embryonal rhabdomyosarcomas of the cervix and other sites, multinodular goiter, differentiated and poorly differentiated thyroid carcinoma, cervical-thyroid teratoma, cystic nephroma-anaplastic sarcoma of kidney, nasal chondromesenchymal hamartoma, intestinal juvenile-like hamartomatous polyp, ciliary body medulloepithelioma, pituitary blastoma, pineoblastoma, primary central nervous system sarcoma, embryonal tumor with multilayered rosettes-like cerebellar tumor, PPB-like peritoneal sarcoma, DICER1-associated presacral malignant teratoid neoplasm and other non-neoplastic associations. Each of these neoplasms is characterized by a second somatic mutation in DICER1. In this review, we have summarized the salient clinicopathologic aspects of these tumors whose histopathologic features have several overlapping morphologic attributes particularly the primitive mesenchyme often with rhabdomyoblastic and chondroid differentiation and an uncommitted spindle cell pattern. Several of these tumors have an initial cystic stage from which there is progression to a high grade, complex patterned neoplasm. These pathologic findings in the appropriate clinical setting should serve to alert the pathologist to the possibility of a DICER1-associated neoplasm and initiate appropriate testing on the neoplasm and to alert the clinician about the concern for a DICER1 mutation.

Proptosis and a Dermal Lesion as the Presenting Sign of Lung Adenocarcinoma

The purpose is to describe a clinical case of orbital metastases as the presentation sign of the primary tumor, and the importance of a multidisciplinary diagnosis. A 70-year-old man attended the Ophthalmology Department referring ocular pain. Mild proptosis and a dermal lesion in the neck were noticed. Biopsy of the dermal lesion and systemic work-up were compatible with lung adenocarcinoma with metastatic dissemination. After one cycle of palliative chemotherapy, patient’s medical condition worsened, and he eventually died. Although rare, orbital symptoms can be the initial clinical presentation preceding the diagnosis of the primary silent lung neoplasm. Ophthalmologist has an important role in diagnoses of metastatic orbital cancer. Involvement of the multidisciplinary team is important for diagnosis and treatment.

Long-term prognostic outcomes in patients with haemoptysis

Background Haemoptysis is a challenging symptom that can be associated with potentially life-threatening medical conditions. Follow-up is key in these patients to promptly detect new or misdiagnosed pathologic findings. Few prospective studies have evaluated long-term prognostic outcomes in patients with haemoptysis. Furthermore, the role played by antiplatelet and anticoagulant drugs on mortality and recurrence rates is unclear. The aim of this study was to assess mortality after 18 months of follow-up. Furthermore, the incidence of recurrence and the risk factors for recurrence and death were evaluated (including the role played by anticoagulant and antiplatelet drugs). Methods Observational, prospective, multicentre, Italian study. Results 451/606 (74.4%) recruited patients with haemoptysis completed the 18 months follow-up. 22/604 (3.6%) diagnoses changed from baseline to the end of the follow-up. 83/604 (13.7%) patients died. In 52/83 (62.7%) patients, death was the outcome of the disease which caused haemoptysis at baseline. Only the diagnosis of lung neoplasm was associated with death (OR (95%CI): 38.2 (4.2–347.5); p-value: 0.0001). 166 recurrences were recorded in 103/604 (17%) patients. The diagnosis of bronchiectasis was significantly associated with the occurrence of a recurrence (OR (95% CI): 2.6 (1.5–4.3)); p-value < 0.0001). Anticoagulant, antiaggregant, and anticoagulant plus antiaggregant drugs were not associated with an increased risk of death and recurrence. Conclusions Our study showed a low mortality rate in patients with haemoptysis followed-up for 18 months. Pulmonary malignancy was the main aetiology and the main predictor of death, whereas bronchiectasis was the most frequent diagnosis associated with recurrence. Antiplatelet and/or anticoagulant therapy did not change the risk of death or recurrence. Follow-up is recommended in patients initially diagnosed with lower airways infections and idiopathic bleeding. Trial registration: NCT02045394

Pulmonary sarcomatoid carcinoma - pathohistological and immunohistochemical analysis, prognosis and complex treatment

The pulmonary sarcomatoid carcinoma (PSC) is extremely rarely lung neoplasm. A woman at the age of 55 with a local advanced pulmonary sarcomatoid carcinoma of the right lung and CT data on bilateral adrenal metastases and three brain metastases were established. Diagnosis is placed after bronchoscopy with biopsy and detailed pathochistological and immunohistochemical analysis. PSC is extremely malignant and with high risk of distant haematogenic metastases. This rare clinical case support the need for strict pathohistological and immunohistochemical analysis, a difficult pathohistological differential diagnosis with other primary malignant lung tumors and the assessment of complex treatment. In order to improve the healing results and survival of patients, timely diagnosis is required at early stage with surgical treatment and subsequent adjuvant chemotherapy and targetеd therapy after genetic analysis of surgery or biopsy tissue material.